ABSTRACT
Haemophilia, an X-linked recessive bleeding disorder, poses a public health challenge globally. In Asia, home to over half the world's population, this challenge is magnified by a factors including immense patient numbers,various socioeconomic diversity.This review examines the complex of pediatric haemophilia across the Asian continent. While high income nations in the region have adopted prophylactic replacement therapy , transforming haemophilia into a manageable chronic condition,it is majorly seen in children in low- and middle-income Asian countries face a identified different reality.
INTRODUCTION
Haemophilia is a congenital coagulopathy characterized by a deficiency in either clotting Factor VIII (haemophilia A) or Factor IX (haemophilia B). This genetic defect, inherited in an X-linked recessive pattern, disrupts the intrinsic pathway of the coagulation cascade, leading to impaired thrombin generation and a lifelong bleeding disorder.For children, the hallmark of the disease is recurrent spontaneous bleeding into synovial joints which triggers a cascade of inflammation, synovial hypertrophy, and cartilaginous destruction. Without adequate intervention, severe pain, and profound disability.The region is a study in contrasts. it includes nations like Japan and South Korea with advanced, government-supported comprehensive care systems, but it is also home to populations with low- and middle-income countries . where the diagnosis of haemophilia itself is a challenge, and access to treatment is a luxury. An estimated 70% of the world's haemophilia population resides in regions with limited access to adequate care, a heavily concentrated in Asia.
METHODS
Many institutions studied and researched to capture region-specific data, reports and publications from the World Federation of Hemophilia (WFH), the Asia-Pacific Hemophilia Network, and national patient organizations were also included.Original research articles published in English within the last 20 years showed significant yet drastic change in the disease.Inclusion criteria focused on studies detailing the epidemiology, diagnosis, clinical management, and outcomes of haemophilia in patients aged 0-18 years within the Asian geographical context.Many countries in central Asia showed noticeable methodological outcome in this disease.
RESULTS
Based on established prevalence rates, Asia is estimated to be home to over 200,000 people with haemophilia. However, the Annual Global Survey reveals a staggering gap between the expected and the diagnosed. In many populous nations like India, Pakistan, Indonesia, and Bangladesh, it is estimated that over 75% of individuals with haemophilia remain not diagnose. This diagnostic means countless boys suffer and die from bleeding complications without ever receiving a specific diagnosis, their condition often relate to other causes or, tragically, non-accidental injury.In high-income nations such as Japan, South Korea, and Singapore, national healthcare systems support primary prophylaxis, starting in early childhood. Many families are forced to make devastating financial choices, and treatment is often delayed or administered at much needed doses, leading to poor outcomes and a higher risk of complications.
DISCUSSION
The management of pediatric haemophilia in Asia is a tale of two . The situation in much of the continent underscores a crisis of access. The prevailing on-demand treatment model in LMICs is not a sustainable healthcare strategy but a reactive measure that perpetuates a cycle of pain, disability, and poverty.Haemophilia management extends beyond infusions, it requires an integrated team of hematologists, pediatricians, orthopedic surgeons, specialist nurses, and physiotherapists. Physiotherapy is particularly critical in resource-limited settings to preserve muscle strength and joint mobility.In conclusion, while the challenges are immense, there is cause for optimism.By prioritizing early diagnosis, pushing for sustainable access to prophylactic treatment, and embracing a holistic model of comprehensive care, it is possible to change the future for children with haemophilia, ensuring that a boy's prognosis is not determined by the country in which he is born.
REFERENCE
https://pubmed.ncbi.nlm.nih.gov
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